Why Are Corneal Specialists Resistant to Treating Patients Who Have Severe Ocular Surface Disease With Limbal Stem Cell Deficiency?

PURPOSE: To highlight the paucity of surgeons performing ocular surface stem cell transplantation with systemic immunosuppression (OSSTx with SI) for limbal stem cell deficiency (LSCD) patients, suboptimal treatments for LSCD, and obstacles to adoption. METHODS: A review of the Eye Bank Association of America annual reports and the authors' case volume for OSSTx with SI was performed. Examination of the published literature on corneal surgeries, especially for LSCD, was completed. These findings were combined with our clinical observations to develop this editorial. RESULTS: Despite techniques and protocols for OSSTx with SI published more than 30 years ago for the treatment of severe bilateral LSCD, only a small number of corneal specialists have adopted these techniques. There is a paucity of attention to this population of patients, with minimal publications to advance this area of our field. We are too often referred patients with LSCD and severe ocular surface disease that have had suboptimal treatments such as penetrating keratoplasties or primary keratoprostheses. Hesitancy for adopting OSSTx with SI is likely due to a lack of exposure to these procedures during training and fear of systemic immunosuppression. Corneal surgeons are likely unaware of the safety of systemic immunosuppression with appropriate monitoring especially when comanaging these patients with an organ transplant specialist. CONCLUSION: There is a large unmet need for the treatment of corneal blindness secondary to conjunctival and LSCD. For the vast majority of patients, OSSTx should be the first surgical choice to treat these eyes. We hope major ophthalmology centers will meet this need by building programs, and groups of corneal surgeons should collaborate to create regional centers to make this treatment more accessible to help this population.

Preparing the Ocular Surface for a Boston Keratoprosthesis Type 1 Through En Bloc Minor Salivary Gland Transplantation and Mucous Membrane Grafting in End-Stage Stevens-Johnson Syndrome.

PURPOSE: This case describes the successful visual restoration of a patient with end-stage Stevens-Johnson syndrome (SJS) with a severely keratinized ocular surface. METHODS: This study is a case report. RESULTS: A 67-year-old man with SJS secondary to allopurinol sought visual rehabilitation options. His ocular surface was severely compromised from sequelae of chronic SJS, leaving him with light perception vision bilaterally. The left eye was completely keratinized with severe ankyloblepharon. The right eye had failed penetrating keratoplasty, limbal stem cell deficiency, and a keratinized ocular surface. The patient declined both a Boston type 2 keratoprosthesis and a modified osteo-odonto keratoprosthesis. Therefore, a staged approach was pursued with (1) systemic methotrexate to control ocular surface inflammation, (2) minor salivary gland transplant to increase ocular surface lubrication, (3) lid margin mucous membrane graft to reduce keratinization, and finally, (4) Boston type 1 keratoprosthesis for visual restoration. After minor salivary gland transplant and mucous membrane graft, the Schirmer score improved from 0 mm to 3 mm with improvement in ocular surface keratinization. This approach successfully restored the vision to 20/60, and the patient has retained the keratoprosthesis for over 2 years. CONCLUSIONS: Sight restoration options are limited in patients with end-stage SJS with a keratinized ocular surface, aqueous and mucin deficiency, corneal opacification, and limbal stem cell deficiency. This case demonstrates successful ocular surface rehabilitation and vision restoration in such a patient through a multifaceted approach that resulted in successful implantation and retention of a Boston type 1 keratoprosthesis.

Interface Fluid Syndrome After Descemet Membrane Endothelial Keratoplasty in Patients With History of LASIK.

PURPOSE: The aim of this study was to evaluate the visual, pachymetric, tomographic, and biomicroscopic findings in a series of cases with laser in situ keratomileusis (LASIK) flap interface fluid syndrome (IFS) after Descemet membrane endothelial keratoplasty (DMEK). METHODS: Six cases were included in this study; all patients had a history of LASIK and underwent DMEK for the treatment of bullous keratopathy. After uneventful surgery, all patients presented with corneal edema and IFS under the LASIK flap, which was demonstrated with anterior segment optical coherence tomography (AS-OCT). Visual acuity, clinical findings, pachymetry, endothelial cell count, and AS-OCT were documented during the management of these cases. RESULTS: IFS appears 2.33 days (±1.03) after DMEK. One case improved with conservative treatment. In 5 cases, the LASIK flap was lifted, the fluid was drained, and the flap was replaced. The mean best-corrected visual acuity after fluid drainage was 0.44 logMAR (range 0.18-1.0) and mean central corneal thickness was 538 µm ± 160. Total resolution of the IFS was achieved at 14.5 days (range 4-30) after DMEK. AS-OCT showed resolution of the flap interface in 5 of 6 cases, while 1 patient required second DMEK due to reaccumulation of the interface fluid. CONCLUSIONS: IFS can occur after DMEK in patients with previous LASIK. AS-OCT is a valuable tool for monitoring these cases preoperatively and postoperatively. Early surgical management is often needed to achieve resolution.

Corneal Pigment Opacity From Face-Down Positioning After Pars Plana Vitrectomy, Treated With Descemet Stripping Only Technique.

PURPOSE: The purpose of this study was to report a case of corneal opacity resulting from pigment deposition after face-down positioning, which was treated with Descemet stripping only (DSO) to enable concurrent pars plana vitrectomy (PPV) for retinal detachment repair. METHODS: A 79-year-old man with a history of Fuchs endothelial dystrophy and retinal detachment presented for the repair of recurrent retinal detachment and evaluation of a central corneal opacity. RESULTS: The patient was found to have significant corneal endothelial pigment deposition obscuring the view to the fundus. A repeat macula-involving retinal detachment was visualized on limited fundoscopic examination and confirmed using ultrasonography. The patient subsequently underwent combination scleral buckle, DSO, and PPV. DSO achieved corneal clarity for the entire duration of the PPV and allowed for the necessary postoperative face-down positioning. Immunohistochemistry of the corneal specimen revealed deposition of retinal pigment epithelium as the origin of the pigment opacity. The corneal edema cleared at postoperative month 4, and the retina remained attached, resulting in an improvement of visual acuity from counting fingers preoperatively to 20/70. DISCUSSION: This is, to the best of our knowledge, the first case describing the formation of a corneal endothelial opacity because of retinal pigment epithelium deposition associated with face-down positioning after PPV for retinal detachment. DSO is a minimally invasive, viable alternative to endothelial keratoplasty or temporary keratoprosthesis placement for the clearance of focal corneal endothelial opacities for PPV.

Animal models of corneal endothelial dysfunction to facilitate development of novel therapies.

Progressive corneal endothelial disease eventually leads to corneal edema and vision loss due to the limited regenerative capacity of the corneal endothelium in vivo and is a major indication for corneal transplantation. Despite the relatively high success rate of corneal transplantation, there remains a pressing global clinical need to identify improved therapeutic strategies to address this debilitating condition. To evaluate the safety and efficacy of novel therapeutics, there is a growing demand for pre-clinical animal models of corneal endothelial dysfunction. In this review, experimentally induced, spontaneously occurring and genetically modified animal models of corneal endothelial dysfunction are described to assist researchers in making informed decisions regarding the selection of the most appropriate animal models to meet their research goals.

Current Global Bioethical Dilemmas in Corneal Transplantation.

PURPOSE: To analyze some of the bioethical dilemmas that may arise during the process required for corneal transplantation. METHODS: We conducted a narrative review based on the available literature and the experience of cornea specialists from 3 different countries. RESULTS: Bioethical dilemmas related to informed consent for organ and tissue donation, allocation of corneal tissues, transplant tourism, corneal tissue exportation and importation, and for-profit eye banking were analyzed and discussed. CONCLUSIONS: Around the world, the number of required corneal transplants exceeds the number of donated corneas that are available and suitable for transplantation. This shortage of corneal tissue has led to the emergence of practices that may put the 4 basic principles of bioethics at risk: autonomy, beneficence, nonmaleficence, and justice. Therefore, it has been necessary to create ethical guidelines such as the Barcelona Principles and the World Health Organization Principles of Transplantation that attempt to regulate these practices.

The Use of Electrotherapeutics in Ophthalmology.

PURPOSE: To present a perspective on the use of electrotherapeutics in the history of ophthalmology along with the development of novel contemporary ophthalmic instrumentation. DESIGN: Perspective study. METHODS: We reviewed historical journals, articles, and books discussing the use of electricity and electrotherapeutics in ophthalmology. RESULTS: Electrotherapeutic applications have been researched and used to treat ocular diseases as far back as the 18th century. By the 20th century, research in electrotherapeutics in ophthalmology had caught the eye of Edward Jackson, the first president of the American Academy of Ophthalmology and Otolaryngology and first editor of the present (third) series American Journal of Ophthalmology. Edward Jackson published an extensive review on this topic and reported a variety of modalities used to treat ocular diseases. CONCLUSIONS: While many early therapeutic uses of electricity did not produce effective and replicable results, studies on electrical stimulation of the eye provided the foundation for the development of clinically significant vision enhancing and restoring instrumentation.

Practice Patterns in the Management of Primary Pterygium: A Survey Study.

PURPOSE: To characterize cornea specialists' current practice preferences in the management of primary pterygium. METHODS: A 25-item survey regarding indications for surgery, surgical technique, use of adjuvant therapy, type and duration of postoperative therapy, and treatment of early recurrences was designed and sent to members of the Cornea Society through the kera-net listserv. RESULTS: In total, 199 cornea specialists completed the questionnaire. More than 90% considered that surgery should be performed when there is proximity of the pterygium to the visual axis, pain or redness, eye movement restriction, or induction of astigmatism. Cosmesis was considered as an indication by 41.7% of the participants. The most frequent technique for pterygium excision was complete resection including the base and a moderate quantity of Tenon capsule followed by autologous conjunctival or limbal-conjunctival graft. The preferred graft fixation method in this survey was fibrin glue (61.2%). Most respondents reported a recurrence rate of less than 5% and no use of adjuvant agents to prevent recurrence. When early recurrence did occur, the preferred agents were corticosteroids. CONCLUSIONS: This study reflects the preferences of cornea experts regarding primary pterygium treatment and may serve as a guide for the management of this pathology.

Epithelial downgrowth after femtosecond laser-assisted cataract surgery.

PURPOSE: To present the case of a 72-year-old female with epithelial downgrowth after femtosecond laser-assisted cataract surgery. OBSERVATIONS: The patient previously underwent YAG vitreolysis after uncomplicated femtosecond laser-assisted cataract surgery and presented 1 year later with epithelial downgrowth causing complete pupillary block and severe angle closure glaucoma. Subsequent management with nd:YAG peripheral iridotomies failed rapidly leading to a confusing presentation with a flat anterior chamber and high intraocular pressure ultimately requiring surgical management. CONCLUSIONS: We describe the occurrence of epithelial downgrowth after femtosecond laser-assisted cataract surgery and illustrate the utility of ultrasound biomicroscopy to differentiate between severe pupillary block and malignant glaucoma.

Posterior Segment Complications and Impact on Long-Term Visual Outcomes in Eyes With a Type 1 Boston Keratoprosthesis.

PURPOSE: To determine the spectrum of retinal complications (RCs) in a cohort of eyes with a type 1 Boston keratoprosthesis (KPro). METHODS: All patients (36 eyes of 31 patients) who received a type 1 Boston KPro from January 2004 to December 2015 at the University of California, Davis, were included. Electronic medical records were reviewed for relevant clinical data. Demographic information, initial corneal diagnosis, postoperative course, posterior segment complications, preoperative and final visual acuity were tabulated and analyzed. RESULTS: Posterior segment complications after type 1 Boston KPro were identified in 56% of eyes (n = 20). They included retinal detachment (n = 11; 31%), retroprosthetic membrane (n = 10; 28%), endophthalmitis (n = 7; 19%), cystoid macular edema (n = 5; 14%), epiretinal membrane (n = 4; 11%), vitreous hemorrhage (n = 2; 6%), choroidal detachment (n = 2; 6%), retinal vein occlusion (n = 1; 3%), and macular hole (n = 1; 3%). During the average follow-up period of 53.8 months (median, 57.1 months; range, 1.8-108.7 months) after type 1 Boston KPro, final best-corrected visual acuity improved by a mean of 0.12 logarithm of the minimum angle of resolution (LogMAR) units (range, -2.26 to +2.26) overall. The proportion of eyes with final best-corrected visual acuity better than 20/200 was 2 of 20 (10%) in the group with RCs, in contrast to 7 of 16 eyes (44%) noted among eyes without RCs. CONCLUSIONS: Long-term visual outcomes in eyes after type 1 Boston KPro may depend, in part, on maintaining a healthy posterior pole. Retinal detachment, in particular, may represent a threat to ultimate visual functioning. Regular examination of the peripheral fundus is recommended.

Effectiveness of Intrastromal Corneal Ring Implantation in the Treatment of Adult Patients With Keratoconus: A Systematic Review.

PURPOSE: To evaluate the available evidence on the effectiveness of intrastromal corneal rings (ICRs) in the treatment of adults with keratoconus. METHODS: A systematic review of electronic databases was completed through July 30, 2017. All primary research articles in which adults with keratoconus were treated with ICRs were included. Two independent reviewers assessed methodological quality and classified the studies into high, low, or undefined risk of bias. The measured variables assessed were visual acuity, refraction, keratometry, ring type, and complications. RESULTS: The initial search yielded 442 scientific articles, 62 articles were read extensively, and 18 articles were assessed for eligibility and included for statistical analysis and quality assessment. A total of 1,325 eyes were analyzed, and the results were evaluated preoperatively and at 12 months of follow-up. Uncorrected distance visual acuity (UDVA) improved 0.23 ± 0.28 logMAR and corrected distance visual acuity (CDVA) improved 0.06 ± 0.21 logMAR. Sphere improved 2.81 ± 1.54 diopters (D), cylinder improved 1.49 ± 0.83 D, and mean keratometry improved 3.41 ± 2.13 D within 12 months of follow-up. ICR implantation combined with corneal crosslinking improved UDVA, refraction, and keratometry to a greater degree than ICR implantation alone. CONCLUSIONS: The studies analyzed demonstrate refractive and visual improvement of patients treated with the ICR implantation technique. However, description of the methodological process necessary to evaluate the bias effectively is insufficient. [J Refract Surg. 2019;35(3):191-200.].

Whorl pattern keratopathies in veterinary and human patients.

The course travelled by corneal epithelial cells from their stem cell niche at the limbus toward the vertex of the cornea is normally not evident due to their transparency, but in certain conditions, the epithelial cells can be rendered visible to the clinician. In such cases, the pathway taken by epithelial cells can manifest as a whorl pattern described using a variety of terms including hurricane keratitis/keratopathy, vortex keratopathy, whorl keratopathy, cornea verticillata, and at times, named after causative agents as exemplified by amiodarone keratopathy. Here, we briefly discuss the terminology used and the spectrum of conditions that can result in keratopathies with whorl patterns in human patients. We review the manifestations of such patterns in veterinary patients and discuss the state of understanding of the underlying forces that create the whorl distribution of epithelial cells on the ocular surface.

Keratoconus and Personality-A Review.

PURPOSE: To assess the existing literature on the subject of keratoconus and personality and to propose a theory that might account for the perceived personality changes associated with this condition. METHODS: A literature search was conducted in the PubMed database using the term "keratoconus" in combination with keywords such as personality, psychiatry, psychology, anxiety, depression, or psychosis. A total of 15 articles pertaining to personality and psychiatric disorders in keratoconus were retained and reviewed. RESULTS: Although patients with keratoconus tend to score differently on personality scales compared with normal controls, the literature fails to substantiate the existence of a unique "keratoconic personality." Instead, patients with keratoconus prove to have more dysfunctional coping mechanisms that specifically alter their interaction with health care providers and may account for the persistent clinical impression of less respectful, conforming, and cooperative patients. CONCLUSIONS: We hypothesize that the stage of life at which keratoconus commonly presents plays a crucial role in personality and coping mechanism development that significantly affects behavioral patterns and the relationship with caregivers.

Favorable Outcome in Coccidioides Endophthalmitis-A Combined Medical and Surgical Treatment Approach.

PURPOSE: To describe a case of Coccidioides endophthalmitis that resulted in a favorable visual outcome after a combined medical and surgical approach. METHODS: A 33-year-old previously healthy woman was referred for evaluation of dyspnea and left-sided vision loss, which began 3 months before, after a trip to Nevada. She was found to have a pulmonary cavitary lesion and fluffy white material in the anterior chamber. An aqueous and vitreous paracentesis grew Coccidioides species. She was managed medically with a total of 7 weekly intravitreal injections of amphotericin B and intravenous liposomal amphotericin B followed by transition to oral posaconazole. Seven months after presentation, to ensure ocular sterilization and to clear the visual axis, she underwent temporary keratoprosthesis implantation, anterior segment reconstruction, removal of a cyclitic membrane and the crystalline lens, pars plana vitrectomy, placement of a pars plana Ahmed drainage device, and penetrating keratoplasty. RESULTS: After surgical intervention and with maintenance posaconazole therapy, the patient had resolution of her dyspnea and improved uncorrected (aphakic) vision with a clear corneal graft, quiet anterior chamber, and normal optic nerve and retina. CONCLUSIONS: A combined medical and surgical approach resulted in a favorable visual outcome and avoided the need for enucleation.

Evaluation of a Portable Artificial Vision Device Among Patients With Low Vision.

IMPORTANCE: Low vision is irreversible in many patients and constitutes a disability. When no treatment to improve vision is available, technological developments aid these patients in their daily lives. OBJECTIVE: To evaluate the usefulness of a portable artificial vision device (OrCam) for patients with low vision. DESIGN, SETTING, AND PARTICIPANTS: A prospective pilot study was conducted between July 1 and September 30, 2015, in a US ophthalmology department among 12 patients with visual impairment and best-corrected visual acuity of 20/200 or worse in their better eye. INTERVENTIONS: A 10-item test simulating activities of daily living was used to evaluate patients' functionality in 3 scenarios: using their best-corrected visual acuity with no low-vision aids, using low-vision aids if available, and using the portable artificial vision device. This 10-item test was devised for this study and is nonvalidated. The portable artificial vision device was tested at the patients' first visit and after 1 week of use at home. MAIN OUTCOMES AND MEASURES: Scores on the 10-item daily function test. RESULTS: Among the 12 patients, scores on the 10-item test improved from a mean (SD) of 2.5 (1.6) using best-corrected visual acuity to 9.5 (0.5) using the portable artificial vision device at the first visit (mean difference, 7.0; 95% CI, 6.0-8.0; P < .001) and 9.8 (0.4) after 1 week (mean difference from the first visit, 7.3; 95% CI, 6.3-8.3; P < .001). Mean (SD) scores with the portable artificial vision device were also better in the 7 patients who used other low-vision aids (9.7 [0.5] vs 6.0 [2.6], respectively; mean difference, 3.7; 95% CI, 1.5-5.9; P = .01). CONCLUSIONS AND RELEVANCE: When patients used a portable artificial vision device, an increase in scores on a nonvalidated 10-item test of activities of daily living was seen. Further evaluations are warranted to determine the usefulness of this device among individuals with low vision.